The Effectiveness of Recombinant Human Erythropoietin (EPO) Treatment in a Neonate with Hyporegenerative Anemia Following Rh Isoimmunization in Spite of Normal Serum Epo Level

Abstract

Postnatal hyporegenerative anemia (PNHA) is characterized by depressed erythropoiesis and low number of erythrocytes. Many mechanisms about PNHA pathogenesis have been implicated: intramedullar hemolysis, bone marrow suppression due to intrauterine transfusions, erythropoetin (Epo) deficiency, erythroid hypoplasia, and erythroid hyporegeneration caused by anti-D antibodies [1–3]. A male baby received intravascular intrauterin transfusions (IIUTs) at the 28th, 29th, 32nd, and 34th gestational weeks and at the 3rd day after delivery due to Rh/Rh isoimmunization. At the 39th day, physical examination revealed paleness and hepatosplenomegaly. Hemoglobin (Hb) was 4.3 g/dL and reticulocytes were 0.6%; leukocytes and thrombocytes were normal. Indirect Coombs test was positive. Liver function and lactate dehydrogenase (LDH) were normal; ferritin was 750 ng/mL. The serologies for viral infections were negative. Bone marrow aspiration revealed erythroid hyperplasia. The patient received packed red blood cells (PRBCs). Hb was 7.6 g/dL and reticulocytes were 0.26% when the patient was readmitted at 50 days old. Occult blood in stool was negative. Other laboratory tests were normal. The patient received PRBCs. The patient was readmitted at 72 days old because of paleness. Hb was 5.4 g/dL and liver function tests increased; LDH was 1447 U/dL. Serum Epo was 220 mIU/mL. The patient received PRBCs. Tocopherol, N-acetylcysteine infusion, and ursodeoxycholic acid were given. Hb decreased to 6.2 g/dL after 5 days following PRBC transfusion. Hyporegenerative anemia was diagnosed and recombinant human erythropoietin (rHuEpo) treatment (200 U/kg thrice a week) was initiated at 88 days old. Liver function tests returned to normal at the 21st day after hospitalization and Nacetylcysteine, tohopherol, and ursodeoxycholic acid were stopped. rHuEpo treatment was stopped at the 47th day of the treatment and his ferritin was 1477 ng/mL and Hb was 11.8 g/dL. At present, the patient is transfusion independent and healthy at 18 months old.